Abstract: Objective : To explore the clinicopathological and radiological features of low-grade central osteosarcoma and to improve the accuracy of its differential diagnosis. Methods : Clinical materials, radiology reports, and histomorphology were analyzed in conjunction with a review of related literature. Results : Histologically, the tumor was characterized by fibroblast-like cell proliferation with irregular anastomosing bone trabeculae formation, resembling fibrous dysplasia or parosteal osteosarcoma. The cells were fairly uniform with mild atypia and low mitotic activity. Conclusion s: Low-grade central osteosarcoma is a well-differentiated subtype of osteosarcoma that is often confused with similar subtypes. Recognition of this variant is based on radiographic findings and careful histologic examination.